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Tubulointerstitial nephritis associated with IgG4-related systemic disease

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Abstract

We report three patients with tubulointerstitial nephritis (TIN) with high serum IgG4 concentrations. None of the patients had notable pancreatic lesions when the TIN developed, although one had a history of autoimmune pancreatitis (AIP). Nevertheless, the clinicopathological findings were quite similar to those of AIP. They were all middle-aged to elderly men. Sialadenitis and lymphadenopathy were often evident. Serum total IgG and IgG4 concentrations were elevated and hypocomplementemia was observed. Although antinuclear antibodies were positive, anti-Ro and anti-La antibodies were negative. Renal biopsy showed dense lymphoplasmacytic infiltration with fibrosis in the renal interstitium, and the infiltrated plasma cells had strong immunoreactivity for IgG4. Furthermore, lymphoplasmacytic infiltration and abundant IgG4-positive plasma cells were observed in the salivary glands of a patient. Steroid therapy was effective for TIN in all three patients. The present findings support the recently proposed concept of IgG4-related systemic disease, and suggest that IgG4 is associated not only with AIP but also with other systemic lymphoplasmacytic diseases, including TIN. The conditions responsible for the pathogenesis of TIN need to be considered, irrespective of the presence of AIP.

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References

  1. SV Seshan VD D'agati GA Appel J Churg (1999) Renal disease. Classification and atlas of tubulo-interstitial and vascular diseases Williams and Wilkins Baltimore

    Google Scholar 

  2. K Okazaki T Chiba (2002) ArticleTitleAutoimmune related pancreatitis Gut 51 1–4 Occurrence Handle1:STN:280:DC%2BD38zjtl2rtA%3D%3D Occurrence Handle10.1136/gut.51.1.1 Occurrence Handle12077078 Occurrence Handle1773283

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. T Kamisawa (2005) ArticleTitleIgG4-related sclerosing disease Internal Med 45 125–6 Occurrence Handle10.2169/internalmedicine.45.0137

    Article  Google Scholar 

  4. T Kamisawa N Egawa H Nakajima K Tsuruta A Okamoto (2005) ArticleTitleExtrapancreatic lesions in autoimmune pancreatitis J Clin Gastroenterol 39 905–7 Occurrence Handle10.1097/01.mcg.0000180629.77066.6c

    Article  Google Scholar 

  5. H Ohhara T Nakazawa H Sano (2005) ArticleTitleSystemic extrapancreatic lesions associated with autoimmune pancreatitis Pancreas 31 232–7 Occurrence Handle10.1097/01.mpa.0000175178.85786.1d

    Article  Google Scholar 

  6. Y Uchiyama-Tanaka Y Mori T Kimura K Sonoyama S Umemura N Kishimoto et al. (2004) ArticleTitleAcute tubulointerstitial nephritis associated with autoimmune-related pancreatitis Am J Kidney Dis 43 18–25 Occurrence Handle10.1053/j.ajkd.2003.12.006

    Article  Google Scholar 

  7. S Takeda J Haratake T Kasai C Takaeda E Takazakura (2004) ArticleTitleIgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis Nephrol Dial Transplant 19 474–6 Occurrence Handle10.1093/ndt/gfg477 Occurrence Handle14736977

    Article  PubMed  Google Scholar 

  8. H Hamano S Kawa A Horiuchi H Unno N Furuya T Akamatsu et al. (2001) ArticleTitleHigh serum IgG4 concentrations in patients with sclerosing pancreatitis N Engl J Med 344 732–8 Occurrence Handle1:CAS:528:DC%2BD3MXitFamt7k%3D Occurrence Handle10.1056/NEJM200103083441005 Occurrence Handle11236777

    Article  CAS  PubMed  Google Scholar 

  9. H Hamano S Kawa Y Ochi H Unno N Shiba M Wajiki et al. (2002) ArticleTitleHydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis Lancet 359 1403–4 Occurrence Handle10.1016/S0140-6736(02)08359-9 Occurrence Handle11978339

    Article  PubMed  Google Scholar 

  10. T Kamisawa N Funata Y Hayashi Y Eishi M Koike K Tsuruta et al. (2003) ArticleTitleA new clinicopathological entity of IgG4-related autoimmune disease J Gastroenterol 38 982–4 Occurrence Handle1:CAS:528:DC%2BD3sXovVKnu7Y%3D Occurrence Handle10.1007/s00535-003-1175-y Occurrence Handle14614606

    Article  CAS  PubMed  Google Scholar 

  11. S Kawa H Hamano (2002) ArticleTitleAssessment of serological markers for the diagnosis of autoimmune pancreatitis (in Japanese, abstract in English) J Japan Pancreas Soc 17 607–10

    Google Scholar 

  12. I Nishimori K Suda I Oi M Ogawa (2002) ArticleTitleResearch on the clinical state of autoimmune pancreatitis (in Japanese, abstract in English) J Japan Pancreas Soc 17 619–27

    Google Scholar 

  13. H Nakamura H Wada T Origuchi A Kawakami N Taura T Aramaki et al. (2006) ArticleTitleA case of IgG4-related autoimmune disease with multiple organ involvement Scand J Rheumatol 35 69–71 Occurrence Handle1:STN:280:DC%2BD28%2FotFOluw%3D%3D Occurrence Handle10.1080/03009740500499484 Occurrence Handle16467047

    Article  CAS  PubMed  Google Scholar 

  14. H Hamano S Kawa Y Ochi N Arakura T Muraki Y Ozaki et al. (2005) ArticleTitleThe pathogenesis of autoimmune pancreatitis J Biliary Tract and Pancreas (Japan) 26 729–36

    Google Scholar 

  15. K Shimoyama N Ogawa T Sawaki H Karasawa Y Masaki H Kawabata et al. (2006) ArticleTitleA case of Mikulicz's disease complicated with interstitial nephritis successfully treated by high-dose corticosteroid Mod Rheumatol 16 176–82 Occurrence Handle10.3109/s10165-006-0478-2 Occurrence Handle16767558

    Article  PubMed  Google Scholar 

  16. M Yamamoto M Harada M Ohara C Suzuki Y Naishiro H Yamamoto et al. (2005) ArticleTitleClinical and pathological differences between Milkulicz's disease and Sjögren's syndrome Rheumatology (Oxford) 44 227–34 Occurrence Handle1:STN:280:DC%2BD2M%2FlsVSlsw%3D%3D Occurrence Handle10.1093/rheumatology/keh447

    Article  CAS  Google Scholar 

  17. M Yamamoto H Takahashi S Sugai K Imai et al. (2005) ArticleTitleClinical and pathological characteristics of Mikulicz's disease (IgG4-related plasmacytic excrinopathy) Autoimunity Rev 4 195–200 Occurrence Handle10.1016/j.autrev.2004.10.005

    Article  Google Scholar 

  18. T Kamisawa N Funata Y Hayashi K Tsuruta A Okamoto K Amemiya et al. (2003) ArticleTitleClose relationship between autoimmune pancreatitis and multifocal fibrosis Gut 52 683–7 Occurrence Handle1:STN:280:DC%2BD3s7ntlGqug%3D%3D Occurrence Handle10.1136/gut.52.5.683 Occurrence Handle12692053 Occurrence Handle1773660

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. S Kitagawa Y Zen K Harada M Sasaki Y Sato H Minato et al. (2005) ArticleTitleAbundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner's tumor) Am J Surg Pathol 29 783–791 Occurrence Handle10.1097/01.pas.0000164031.59940.fc Occurrence Handle15897744

    Article  PubMed  Google Scholar 

  20. T Parlowsky J Welzel M Amagai D Zillikens T Wygold (2003) ArticleTitleNeonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborn J Am Acad Dermatol 48 623–5 Occurrence Handle10.1067/mjd.2003.170 Occurrence Handle12664033

    Article  PubMed  Google Scholar 

  21. SJ Warren LA Arteaga EA Rivitti V Aoki G Hans-Filho BF Qaqish et al. (2003) ArticleTitleThe role of subclass switching in the pathogenesis of endemic pemphigus foliaceus J Invest Dermatol 120 104–8 Occurrence Handle1:CAS:528:DC%2BD3sXnvFCgtg%3D%3D Occurrence Handle10.1046/j.1523-1747.2003.12017.x Occurrence Handle12535205

    Article  CAS  PubMed  Google Scholar 

  22. RC Aalberse F Van Milligen KY Tan SO Stapel (1993) ArticleTitleAllergen-specific IgG4 in atopic disease Allergy 48 559–69 Occurrence Handle1:STN:280:DyaK2c7mtFamtA%3D%3D Occurrence Handle10.1111/j.1398-9995.1993.tb00749.x Occurrence Handle8116855

    Article  CAS  PubMed  Google Scholar 

  23. T Muraki H Hamano Y Ochi K Komatsu Y Komiyama N Arakura et al. (2006) ArticleTitleAutoimmune pancreatitis and complement activation system Pancreas 32 16–21 Occurrence Handle10.1097/01.mpa.0000188308.75043.e4 Occurrence Handle16340739

    Article  PubMed  Google Scholar 

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Correspondence to Takako Saeki.

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Saeki, T., Saito, A., Yamazaki, H. et al. Tubulointerstitial nephritis associated with IgG4-related systemic disease. Clin Exp Nephrol 11, 168–173 (2007). https://doi.org/10.1007/s10157-007-0464-9

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  • DOI: https://doi.org/10.1007/s10157-007-0464-9

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